Chorea-acanthocytosis (ChAc) is a rare, severe, and progressive hereditary neurodegenerative disease with a poor prognosis. The patient in this case was a 29-year-old female who presented with gradually worsening behavioral abnormalities and slurred speech as the main symptoms. Gene sequencing revealed a mutation in the VPS13A gene, leading to a diagnosis of"Chorea-acanthocytosis". This case report aims to improve the awareness among psychiatrists in distinguishing movement disorders and behavioral disorders. Combined with a literature review on the clinical manifestations, differential diagnosis, and genetic testing, this paper may help psychiatrists in early identification and accurate diagnosis of ChAc.

ObjectiveTo investigate the prevalence and influencing factors of overweight， obesity and central obesity among elderly residents in Xuhui District， and to analyze the epidemiological status of central obesity in elderly people with different body mass indexes. MethodsThe third round of health status and health service utilization monitoring data in Xuhui District was used. The information collected from questionnaire survey and physical examination were analyzed. SPSS 20.0 software was used for χ² test， trend χ² test and multinominal logistic regression analysis. Results5 096 survey subjects were included. The prevalence of overweight， general obesity， and central obesity in the residents aged 60 and above in Xuhui District were 34.3%， 6.5%， and 29.2%， respectively. There was gender difference in the rates of overweight， obesity， and central obesity among the residents. The overweight and central obesity rates in males were higher than those in females， while the obesity rate was lower than that in females （P<0.05）. Multinominal logistic regression analysis showed that in comparison with the normal weight non-central obesity group drinking only at party （OR=1.729， 95%CI： 1.184‒2.525）， and hypertension （OR=1.637， 95%CI： 1.305‒2.053）， were highly associated with normal weight with central obesity. Aged 60‒ years （OR=1.589， 95%CI： 1.190‒2.120）， aged 70‒ years （OR=1.763， 95%CI： 1.327‒2.342）， male （OR=1.379， 95%CI： 1.134‒1.676）， hypertension （OR=2.231， 95%CI： 1.878‒2.649）， former smokers （OR=1.437， 95%CI： 1.027‒2.011）， drinking at party only （OR=1.491， 95%CI： 1.107‒2.006）， and drinking ≥3 times per week （OR=1.611， 95%CI： 1.116‒2.325）， were highly associated with overweight combined with central obesity. Aged 60‒ years （OR=3.817， 95%CI： 2.251‒6.474）， aged 70‒ years （OR=3.084， 95%CI： 1.838‒5.175）， hypertension （OR=3.683， 95%CI： 2.753‒4.929）， diabetes （OR=2.085， 95%CI： 1.511‒2.878）， former smokers （OR=1.835， 95%CI： 1.043‒3.226）， were highly associated with compound obesity. Central obesity was found in the elderly residents with different BMI categories， and the rate of central obesity increased with the increase of BMI grade. ConclusionThe prevalence of overweight， obesity and central obesity among the elderly aged 60 years and above in Xuhui District is not optimistic. Attention should be paid to the elderly under 80 years old who are with hypertension and/or diabetes， alcohol consumption， low educational level and not doing physical exercise. Especially for the central obesity population with normal BMI， measures should be taken to prevent and intervene the occurrence of obesity and related diseases.

Chorea-acanthocytosis (ChAc) is a rare, severe, and progressive hereditary neurodegenerative disease with a poor prognosis. The patient in this case was a 29-year-old female who presented with gradually worsening behavioral abnormalities and slurred speech as the main symptoms. Gene sequencing revealed a mutation in the VPS13A gene, leading to a diagnosis of"Chorea-acanthocytosis". This case report aims to improve the awareness among psychiatrists in distinguishing movement disorders and behavioral disorders. Combined with a literature review on the clinical manifestations, differential diagnosis, and genetic testing, this paper may help psychiatrists in early identification and accurate diagnosis of ChAc.

Objective To compare the success rate and complication rate between real-time ultrasound-guided and technique modified methods of right subclavian vein puncture in tumor patients during implantation of totally implanted port, and to evaluate their effectiveness and safety. Methods We randomly divided 260 tumor patients into ultrasound-guided group and technique modified group, and compared the success rate, pneumothorax, arterial injury, ectopic catheter, local hematoma, puncture times, pinch-off syndrome, etc. Results The success rate of primary puncture in the ultrasound-guided group was better than that in the technique modified group (96.92% vs. 86.15%, P=0.002). The overall complication of the ultrasound-guided group was lower than that of the technique modified group (0 vs. 9.23%, P < 0.001). The incidence of pneumothorax (0 vs. 3.85%, P=0.024) and accidental arterial injury (0 vs. 3.08%, P=0.044) in the ultrasound-guided group were lower than those in the technique modified group. There was no arteriovenous fistula and pinch-off syndrome in any group. Conclusion Compared with the technique modified group, the ultrasound-guided group has more advantages in puncture. Patients with vascular variability can be detected in time.

Objective To analyze the clinical efficacy,safety and influencing factors of decitabine (DAC)-based regimens in patients with myelodysplastic syndrome-refractory anemia with excess blasts (MDS-RAEB).Methods We performed a retrospective analysis of 63 patients with MDS-RAEB treated with DAC,evaluated the clinical efficacy and adverse reactions,and analyzed the influencing factors affecting survival.Results Among 63 patients,23 were RAEB-1 and 40 were RAEB-2.The median treatment was 4 (2-13) courses.The total effective rate of DAC for MDS-RAEB was 58.7％ (37/63),and the complete response rate was 20.6％ (13/63).Among 37 patients who were effective,20 (54.1％) patients performed efficacy after 2 courses.The median course of treatment to achieve the best effect was 3.5 (3-4) courses.With a median follow-up of 14 (2-68) months,63 patients had a overall survival rate (OS) of 84.2％ and a 1-year progression-free survival rate (PFS) of 73％.In univariate analysis,the factors that prolonged OS were that the best effect after medication was stable disease (SD) (to achieve complete remission,partial remission,complete bone marrow remission,hematological improvement) (P =0.009) and no thrombocytopenia at first diagnosis (P =0.019),the factor that prolongs PFS is the best effect above SD (P =0.003).Multivariate analysis suggested that the factors affecting OS and PFS were the best curative effects above SD (P =0.015 vs P =0.008).The adverse effects of decitabine in the treatment of MDSRAEB were mainly bone marrow suppression and pulmonary infection.Conclusions Decitabine is an effective and well-tolerated drug in the treatment of MDS-RAEB.Response to decitabine treatment is one of the independent factors affecting the prognosis.

Objective To investigate the efficacy of domestic porcine antihuman lymphocyte immunoglobulin (p-ALG) in the treatment of severe aplastic anemia (SAA) with allogeneic hemopoietic stem cell transplantation (HSCT). Methods The clinical data of 5 SAA patients who received allogeneic HSCT from January 2015 to May 2018 were retrospectively analyzed. The conditioning regimen included p-ALG + cyclophosphamide + fludarabine. The method of peripheral stem cell and bone marrow blood was used in allogeneic HSCT (the total amount of bone marrow blood was less than 400 ml and the puncture point was not replaced). The p-ALG related complications, post-transplantation hemopoietic stem cell reconstitution time and efficacy were recorded. Results Allergic reaction occurred in 1 patient when using p-ALG, and there was no serum reaction. Hemopoietic reconstitution was achieved in all the 5 patients. The time for neutrophilic granulocyte > 0.5 × 109/L was 11 to 17 d, and the time for platelet count > 20 × 109/L was 11 to 15 d after transplantation. The results of short-strand repeat polymerase chain reaction assays showed all complete donor chimera. Graft versus host disease occurred in 3 cases, and was successfully controlled by methylprednisolone and tacrolimus. The time for stopping red blood cell transfusion was 9 to 87 d. The patients were followed up for 1 to 37 months, and the patients all survived well. Conclusions The efficacy of p-ALG in SAA patients of allogeneic HSCT is affirmative, and the cost is obviously reduced. It is worthy of clinical use.

Objective To investigate the safety,effectiveness and prognosis of percutaneous microwave ablation (MWA) combined with synchronous transarterial chemoembolization (TACE) to treat of gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) with liver metastases (LMs).Methods This retrospective study included 19 cases of GEP-NENs with LMs patients who received percutaneous MWA combined with synchronous TACE treatment from 2013 to 2016.The mRECIST standard was selected to assess the curative effect.SPSS 21.0 software was applied in the statistical analysis of overall survival (OS),progression-free survival (PFS) and factors related to prognosis.Results All patients were capable of curative effect evaluation,including 1 case of complete remission (CR),3 cases of partial remission (PR),7 cases of progressive disease (PD) and 8 cases of stable disease (SD) respectively accounting for 5 ％,16 ％,37 ％,42 ％,which exhibited 21％ of response rate (RR) and 63％ disease control rate (DCR).In the present study,the median OS and median PFS was respectively 25 months and 34 months,and the one-year survival and three-year survival was respectively 95％ and 84％.Serum CA199,the WHO classification of LMs and the tumor burden of LMs were the major risk factors of prognosis through single factor analysis of survival,which showed that G3 of the WHO classification of LMs predicted a poor OS (P＜0.05) and tumor burden of LMs was negatively related to PFS (P＜0.05).It was obviously observed that serum CgA was decreased by the therapy of percutaneous MWA with synchronous TACEfor GEP-NENs (P＜0.05).Conclusions Percutaneous MWA combined with synchronous TACE is a safe and effective method to treat GEP-NENs with LMs.

Objective To investigate the efficacy, safety and prevention of complications of haploidentical allogeneic hematopoietic stem cell transplantation (Haplo-HSCT) in patients with severe aplastic anemia (SAA). Methods The clinical data of 8 SAA patients treated with Haplo-HSCT were retrospectively analyzed, with male in 5 cases, female in 3 cases, and an average age of 14 years. The donors of hematopoietic stem cells were patient′ s parents. The preparative regimen was cyclophosphamide + fludarabine + anti-human lymphocyte immune globulin, and 2 patients combined with total body irradiation. The recipients received cyclosporine, short- term methotrexate and mycophenolate mofetil (MMF) for preventing graft versus host disease (GVHD). The hematopoietic reconstitution, the chimerism rate of donor cells, and the incidence of postoperative complications such as infection, GVHD, liver veno- occlusive disease (VOD), hemorrhagic cystitis (HC), and cytomegalovirus (CMV) were observed. Results Hematopoietic reconstitution was achieved in all the 8 patients, the neutrophil engraftment time was (14.80 ± 3.24) d, and the platelet engraftment time was (15.00 ± 3.42) d. One month after transplantation, all the patients had complete DNA chimerism. Seven patients occurred acute GVHD, includingⅠ-Ⅱgrade in 5 cases, Ⅲgrade in 1 case, andⅣgrade in 1 case. Two patients occurred chronic GVHD, including 1 case with localized GVHD in the oral cavity and 1 case with extensive type chronic GVHD in the whole body skin. No liver VOD or HC occurred. No transplantation-related death occurred at average follow-up time of 8.5 (2 - 18) months. Conclusions Haplo-HSCT is safe and effective in patients with SAA.

Objective To investigate and analyze the incidence of onychomycosis coupled with diabetes in the inpatients, the clinical classification of the disease and species composition.Methods Two hundred and seven patients with diabetes admitted into our hospital from June 2014 to December 2014, were used as the research objects.The incidence of onychomycosis, the clinical classifica-tion of the disease and species composition were investigated and analyzed in the inpatients coupled with diabetes.Results The clinical classifications of onychomycosis in 207 inpatients coupled with diabetes were DLSO (accounting for 42.9%), TDO (accounting for 38.1%), PSO (accounting for 14.3%) and SWO (accounting for 4.8%).The incidence of onychomycosis in diabetic inpatients was 10.1%, with the main pathogens of the disease being dermatophyte and yeast.Conclusion The incidence of onychomycosis in diabetic inpatients was higher than that of the general population.Clinical classification of the disease was chiefly DLSO, with pathogens being dermatophyte and yeast.

Objective To investigate the role of prenatal single-dose administration of dexamethasone and ambroxol on the expression of Toll-like receptor 4 (TLR4) of fetal and neonatal rats. Methods Fifty-four pregnant rats were randomly divided into three groups with eighteen rats in each group:rats treated with 0.2 mg/kg dexamethasone (group 1),0.2 mg/kg dexamethasone and 100 mg/kg ambroxol (group 2),or saline(controls) on the 17th day of gestation.The lung tissues of the offsprings were harvest independently on the 19th day of gestation,the postnatal 3 days and 7 days.The expressions of TLR4 in fetal/neonatal rat lungs of each pregnant rat were analyzed by reverse transcription-polymerase chain reaction(RT-PCR),immunohistochemistry stain,and Western blot. ANOVA and two independent samples t-test were applied. Results On the 19th day of pregnancy,TLR4 mRNA expression was up-regulated in lungs of the two treatment groups compared with controls(controls:0.26 ± 0.18,group 1:0.39 ± 0.21,t =5.866,P＜ 0.05 ; control:0.27 ± 0.22,group 2:0.46 ± 0.13,t =9.572,P＜ 0.01 ).TLR4 mRNA expression was up-regulated in group 2 compared with controls on the postnatal 3 days and 7 days(postnatal 3 d:0.59 ± 0.23 and 0.47 ±0.24,t=2.295,P＜0.05;postnatal 7 d:0.52±0.12 and 0.35±0.17,t=4.219,P＜0.05),while no significant difference was found in group 1 compared with the controls(postnatal 3 d:0.45±0.22 and 0.44±0.14,t=0.128,P＞0.05; postnatal 7 d:0.40±0.16 and 0.36 ±0.12,t=1.365,P＞0.05).Results of the immunohistochemistry demonstrated that on the 19th day of pregnancy,the protein expression of TLR4 was significantly increased in the two treatment groups (controls:0.20 ± 0.29,group 1:0.35±0.32,t=7.179,P＜0.05 ;controls:0.20±0.29,group 2:0.39±0.25,t=10.764,P＜0.01).The protein expression of TLR4 was significantly increased in group 2 on the postnatal 3 days and 7 days(postnatal 3 d:0.55±0.32 and 0.37±0.18,t=7.121,P＜0.05;postnatal 7 d:0.41±0.29and 0.25±0.24,t=6.355,P＜0.05),while no notable difference was found between group 1 and the control (postnatal 3 d:0.40±0.21 and 0.37±0.18,t=0.683,P＞0.05 ;postnatal 7 d:0.28±0.31 and 0.25±0.24,t=0.462,P＞0.05).Results of the Western blot demonstrated that on the 19th day of pregnancy,the protein expression of TLR4 was significantly increased in the two treatment groups (controls:0.15 ± 0.12,group 1:0.27± 0.20,t =7.835,P＜0.05; controls:0.16 ± 0.18,group 2:0.34±0.16,t=10.470,P＜0.01).The protein expression of TLR4 was significantly increased in lungs of the combination administration group on the postnatal 3 days and 7 days(postnatal 3 d:group 2:0.37±0.20 and 0.25±0.22,t=6.379,P＜0.05; postnatal 7 d:0.35±0.15 and 0.24±0.13,t=5.152,P＜0.05),while no notable difference could be found between group 1 and the control (postnatal3 d:0.32±0.26 and 0.25±0.16,t=1.167,P＞0.05; postnatal 7 d:0.29±0.19 and 0.24±0.10,t =1.248,P ＞ 0.05 ). Conclusions Prenatal single-dose administration of dexamethasone may up-regulate the expression of TLR4 in the rat fetal lung.The up-regulation of TLR4 might be one of the critical factors for glucocorticoid-induced maturity of fetal lung.Prenatal single-dose administration of dexamethasone and ambroxol may have effects on the regulation of TLR4 not only in fetal rats,but also in neonatal rats.